Benign tumors of the ampulla of Vater are rare. The villous adenoma, the most common benign tumor of this region, has a high tendency to malignant transformation. Postmortem studies showed an incidence of 0.04-0.12% adenomatous lesions of the papilla of Vater. The authors present a case of villous adenoma of the ampulla of Vater and make a review of the literature.